Kong Kam Wa T, Holmes C, O'Brien K
Department of Anaesthesiology Children's Health Ireland at Temple Street Dublin Ireland.
Anaesth Rep. 2021 Nov 17;9(2):e12138. doi: 10.1002/anr3.12138. eCollection 2021 Jul-Dec.
Spinal muscular atrophy is a neuromuscular disorder with degeneration of spinal motor neurons. Type I is a severe variant that was recently shown to be amenable to treatment with the antisense oligonucleotide nusinersen. As a result of increased life expectancy with this treatment, more children with spinal muscular atrophy type I are presenting for spinal correction surgery. In this case series, we present four such patients who underwent spinal surgery at our institution over the course of one year. Pre-operative assessment showed evidence of reduced respiratory function requiring nocturnal non-invasive ventilation in all four patients. A difficult airway was encountered in two of the four patients. Postoperative complications were ubiquitous and included CSF leak, poor wound healing, metal frame exposure, frame instability and wound infection. There were no postoperative respiratory complications and all four children returned to their respiratory baseline postoperatively. All patients underwent successful lumbar puncture and intrathecal nusinersen injection following their spinal surgeries. Given the risk of complications and prolonged recovery following spinal surgery, a detailed family discussion is advisable.
脊髓性肌萎缩症是一种伴有脊髓运动神经元变性的神经肌肉疾病。I型是一种严重的变体，最近显示可采用反义寡核苷酸诺西那生进行治疗。由于这种治疗方法提高了预期寿命，越来越多的I型脊髓性肌萎缩症患儿前来接受脊柱矫正手术。在这个病例系列中，我们介绍了在一年时间里于我们机构接受脊柱手术的四名此类患者。术前评估显示，所有四名患者均有呼吸功能下降的证据，需要夜间无创通气。四名患者中有两名遇到了困难气道。术后并发症普遍存在，包括脑脊液漏、伤口愈合不良、金属框架外露、框架不稳定和伤口感染。术后没有呼吸并发症，所有四名儿童术后均恢复到呼吸基线水平。所有患者在脊柱手术后均成功进行了腰椎穿刺和鞘内注射诺西那生。鉴于脊柱手术后存在并发症风险和恢复时间延长的情况，建议进行详细的家庭讨论。
